Nebulin

NEB
Available structures
PDBHuman UniProt search: PDBe RCSB
Identifiers
AliasesNEB, nebulin, NEB177D, NEM2, AMC6
External IDsOMIM: 161650; MGI: 97292; HomoloGene: 136285; GeneCards: NEB; OMA:NEB - orthologs
Orthologs
SpeciesHumanMouse
Entrez

4703

17996

Ensembl

ENSG00000183091

ENSMUSG00000026950

UniProt

P20929
Q05C45

n/a

RefSeq (mRNA)

NM_001164507
NM_001164508
NM_001271208
NM_004543

NM_010889

RefSeq (protein)

NP_001157979
NP_001157980
NP_001258137
NP_004534
NP_004534.2

n/a

Location (UCSC)Chr 2: 151.49 – 151.73 MbChr 2: 52.14 – 52.38 Mb
PubMed search
Wikidata
View/Edit HumanView/Edit Mouse

Nebulin is an actin-binding protein which is localized to the thin filament of the sarcomeres in skeletal muscle. Nebulin in humans is coded for by the gene NEB. It is a very large protein (600–900 kDa) and binds as many as 200 actin monomers. Because its length is proportional to thin filament length, it is believed that nebulin acts as a thin filament "ruler" and regulates thin filament length during sarcomere assembly. Other functions of nebulin, such as a role in cell signaling, remain uncertain.

Nebulin has also been shown to regulate actin-myosin interactions by inhibiting ATPase activity in a calcium-calmodulin sensitive manner.

Mutations in nebulin cause some cases of the autosomal recessive disorder nemaline myopathy.

A smaller member of the nebulin protein family, termed nebulette, is expressed in cardiac muscle.